Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) in association with an adenocarcinoma: a case report

Arne Warth,Konstantina Storz,Philipp A Schnabel,Astrid Schmähl,Esther Herpel

doi:10.1186/1752-1947-2-21

Abstract

IntroductionDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare disorder and information on this disease is limited, especially with regard to its management and prognosis. It has become generally accepted that DIPNECH is a precursor lesion to pulmonary carcinoid tumors.Case presentationHere we report on a 60-year-old female patient with DIPNECH and an associated pulmonary adenocarcinoma.ConclusionThis case contributes to a better understanding of the disorder and its associated pathologies.

Highlights

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare disorder and information on this disease is limited, especially with regard to its management and prognosis
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is an exceedingly rare disorder and only 40 cases have been described in the literature to date [1]
According to the current WHO classification, this disorder is characterized by one of the following: a generalized proliferation of scattered single cells, small nodules or linear proliferations of pulmonary neuroendocrine cells [2]; in addition, it is considered to be a precursor for pulmonary carcinoid tumors

Summary

Introduction

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is an exceedingly rare disorder and only 40 cases have been described in the literature to date [1]. Further processing of the multiple small lesions in the upper and middle lobe revealed five foci less than 5 mm in diameter with a different trabecular and nest-like morphology (Fig. 2) In these lesions, the cells were strongly positive for CD56, synaptophysin, NSE and chromogranin A and focally positive for CK7, CK18, TTF1 with a proliferation rate (Ki67) of 1–2%. The current report represents the first case of a patient with DIPNECH accompanied by a pulmonary adenocarcinoma of mixed subtype with partial neuroendocrine differentiation. It is unlikely that DIPNECH is a precursor lesion for other tumors of the lung with neuroendocrine differentiation besides carcinoid tumors [6], this possibility cannot yet be excluded considering the small number of the cases described to date.

Kerr KM

Gosney JR

Conclusion